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Epithelioid sarcoma is a rare variety of soft tissue sarcoma that affects young adults mostly involves forearm and hand. It mainly presents as a painless slow growing mass, but can present as multifocal lesions. It has a high rate of recurrence and metastasis. A 35-year-old male presented to our department in November 2013 with a painful ulcer with bloody discharge in the medial side of left wrist. The patient gave a history of swelling with serous discharge from 11 years. Local excision was done 6 times from 2003 to 2011. The patient also gave a history of falling on an outstretched hand and swelling at same site in July 2013, this time again wide local excision was done. The histopathology report was suggestive of high-grade osteogenic sarcoma. Immunohistochemistry (IHC) showed expression of cytokeratin, epithelial membrane antigen, CD34 and CA-125; tumor cells had lost INI-1 expression. IHC confirmed the diagnosis of epithelioid sarcoma of left wrist. Contrast-enhanced computed tomography thorax was suggestive of lung metastasis. Thus, doxorubicin based chemotherapy was planned.
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